Juvenile Systemic Sclerosis/discoid Lupus Erythematosus Overlap Syndrome: A Case Report
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Abstract
Juvenile systemic sclerosis is rare and the association of systemic sclerosis with discoid lupus erythematosus is uncommon. To date, no juvenile systemic sclerosis with discoid lupus erythematosus has been reported. We described a case of 15-year-old girl diagnosed as juvenile diffuse systemic sclerosis/discoid lupus erythematosus overlap syndrome with initial presentation of Raynaud’s phenomenon, digital pitted scar, and skin sclerosis. She developed multiple atrophic scars on her both dorsum of hands and wrists two years later. Skin histopathology showed vacuolar degeneration of basal cell layer, superficial and deep perivascular lymphoplasmacytic infiltration, together with periadnexal inflammation. In addition, sclerosis of collagen is also identified. Direct immunofluorescence revealed C3, IgA, IgG, and IgM deposition along dermal-epidermal junction in granular pattern. Antinuclear antibodies and anti topoisomerase-I antibody were positive, while anti-doublestranded DNA, anti-U1RNP were negative. The patient fulfills the criteria for juvenile systemic sclerosis, while histopathology and direct immunofluorescence revealed finding consistent with discoid lupus erythematosus. Chest radiography and transthoracic echocardiogram were normal. The treatment was started with antimalarials, antifibrotic agents, calcium channel blocker, antiplatelet and topical corticosteroid. There was improvement in skin sclerosis and Raynaud’s phenomenon during 2-year follow up.
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References
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